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NCCN Guidelines for Patients Nasopharyngeal Cancer

NCCN Guidelines for Patients Nasopharyngeal Cancer

our Pleaseonline completesurvey at NCCN.org/patients/survey

NCCN GUIDELINES FOR PATIENTS® 2019 Nasopharyngeal HEAD AND NECK SERIES

Presented with support from: Support for People with Oral and

Available online at NCCN.org/patients Ü Nasopharyngeal Cancer

It's easy to get lost in the cancer world

Let  NCCN Guidelines for Patients® be your guide

9 Step-by-step guides to the cancer care options likely to have the best results

9 Based on treatment guidelines used by health care providers worldwide

9 Designed to help you discuss cancer treatment with your doctors

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 1 About

NCCN Guidelines for Patients® are developed by the National Comprehensive Cancer Network® (NCCN®)

NCCN Clinical Practice ®   NCCN Guidelines NCCN Guidelines in ® ® for Patients (NCCN Guidelines )

 An alliance of 28 leading  Developed by doctors from  Present information from the cancer centers across the NCCN cancer centers using NCCN Guidelines in an easy- devoted to the latest research and years to-learn format patient care, research, and of experience education  For people with cancer and  For providers of cancer care those who support them all over the world Cancer centers  Explain the cancer care that are part of NCCN:  Expert recommendations for options likely to have the NCCN.org/cancercenters cancer screening, diagnosis, best results and treatment

Free online at Free online at NCCN.org/patientguidelines NCCN.org/guidelines

and supported by funding from NCCN Foundation®

These NCCN Guidelines for Patients® are based on the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Head and Neck Cancers (Version 3.2019, September 16, 2019).

© 2019 National Comprehensive Cancer Network, Inc. All rights reserved. NCCN Foundation® seeks to support the millions of patients and their families NCCN Guidelines for Patients® and illustrations herein may not be reproduced affected by a cancer diagnosis by funding and distributing NCCN Guidelines for in any form for any purpose without the express written permission of NCCN. No Patients®. NCCN Foundation is also committed to advancing cancer treatment one, including doctors or patients, may use the NCCN Guidelines for Patients for by funding the nation’s promising doctors at the center of innovation in cancer any commercial purpose and may not claim, represent, or imply that the NCCN research. For more details and the full library of patient and caregiver resources, Guidelines for Patients that have been modified in any manner are derived visit NCCN.org/patients. from, based on, related to, or arise out of the NCCN Guidelines for Patients. The NCCN Guidelines are a work in progress that may be redefined as often National Comprehensive Cancer Network® (NCCN®) / NCCN Foundation® as new significant data become available. NCCN makes no warranties of any 3025 Chemical Road, Suite 100 kind whatsoever regarding its content, use, or application and disclaims any Plymouth Meeting, PA 19462 responsibility for its application or use in any way. 215.690.0300

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 2 Supporters

Supported by

Head and Neck Cancer Alliance As an organization that works to advance prevention, detection, treatment and rehabilitation of oral, head and neck cancers, the Head and Neck Cancer Alliance strongly supports and endorses these NCCN Guidelines for Patients. headandneck.org

Oral Cancer Foundation The foundation is pleased to support and endorse the NCCN Guidelines for Patients. These guidelines provide another tool for patients to make sound science-based decisions from as they navigate the treatment options and journey through the cancer experience. As an organization that works to provide vetted information to patients and caregivers, sponsor research, advocate for issues that impact this patient population, and provide real time support for them from both an emotional support and science based treatment perspective, we understand that sound factual knowledge is empowering. It ultimately is a key component of decision making that yields better long-term outcomes. We appreciate having another powerful tool in the armamentarium available to them. oralcancerfoundation.org

Support for People with Oral and Head and Neck Cancer (SPOHNC) SPOHNC is honored to support and endorse the new NCCN Guidelines for Patients.

Support for People with Together we can help to raise awareness, educate and support patients with the Oral and Head and Neck Cancer necessary tools that will guide them while choosing their treatment options and managing their care. Together we are committed to offering oral, head and neck cancer patients and their loved ones the best support services available to live a better quality of life from diagnosis through recovery. spohnc.org

With generous support from Endorsed by • André Marc DuPont in honor of Edward King THANC- The THANC Foundation is pleased to endorse • Robert Foote, MD, FACR, FASTRO this valuable resource. We believe this comprehensive • Steven Frank, MD guide will help patients understand and prepare for • Ying Hitchcock, MD every step of the head and neck cancer journey. thancfoundation.org • Bharat B. Mittal, MD • Lisle Marie Nabell, MD • Dr. and Mrs. David Pfister The Thyroid Care Collaborative strongly endorses this • Sharon Spencer, MD valuable resource. We believe this comprehensive guide will ensure patients are well-informed - an important step • Weining Zhen, MD to the fight against head and neck cancer! thyroidccc.org

To make a gift or learn more, please visit NCCNFoundation.org/donate or email [email protected]

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 3 NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 4 Nasopharyngeal Cancer

Contents

6 Nasopharyngeal cancer basics

12 Testing for nasopharyngeal cancer

20 Treating nasopharyngeal cancer

33 Making treatment decisions

42 Words to know

44 NCCN Contributors

45 NCCN Cancer Centers

46 Index

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 5 1 Nasopharyngeal cancer basics

7 The throat 8 Head and neck cancer 9 Risk factors 9 Treatment types 11 Review

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 6 1 Nasopharyngeal cancer basics The throat

Nasopharyngeal cancer is a type of Air also passes from either the mouth or nose throat cancer. Read this chapter to learn through the on its way to the lungs. where this cancer grows and what puts people at risk for this cancer. The pharynx has 3 parts, which are the:

† nasopharynx,

† oropharynx, and

The throat † hypopharynx.

Before learning about nasopharyngeal cancer, The nasopharynx is behind the nose. The it is helpful to know about the throat. The throat oropharynx is below the nasopharynx. The is also called the pharynx. hypopharynx is below the oropharynx and just behind the voice box. See Figure 1. Pharynx The pharynx is a tube-like structure within the Lymph head. It starts behind the nose and ends at the Throughout your body—including your throat— voice box (). Food passes from the mouth is a clear fluid called lymph. Lymph contains through the pharynx on its way to the stomach. food and water for your cells. It also contains germ-fighting blood cells. Lymph drains from

Figure 1 The throat

The throat is also called the pharynx. It has three parts. Behind the nose is the nasopharynx. Below the nasopharynx is the oropharynx then the hypopharynx.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 7 1 Nasopharyngeal cancer basics Head and neck cancer

body tissues into channels that transport it to Head and neck cancer the bloodstream. Head and neck cancer is a group of cancers. It As lymph travels, it passes through lymph includes cancers of the inner , mouth, throat, tissue. Lymph tissue removes germs from voice box, nose, and sinuses. These cancers lymph. Lymph nodes are small structures are referred to as upper aerodigestive tract that contain lymph tissue. They are found cancers. throughout the body. There are many lymph nodes in the left and right sides of the neck. Nasopharyngeal See Figure 2. This book is about treatment for nasopharyngeal carcinoma. Almost all The tonsils are larger clusters of lymph tissue. nasopharyngeal cancers are . There is a tonsil on the left and right side of Carcinomas are cancers of cells that line the the throat. There are similar clusters of lymph inner or outer surfaces of the body. tissue in the nasopharynx and at the back of the tongue. Nasopharyngeal carcinoma forms from cells that line the nasopharynx. The cancer cells can grow into nearby tissue, such as the skull. They often spread to lymph nodes behind the ears and in the neck.

Figure 2 Lymph nodes

Throughout your body is a clear fluid called lymph. It drains from tissues into vessels that carry it back to the bloodstream. As lymph travels, it passes through small structures called lymph nodes. Lymph nodes remove germs from lymph. Lymph vessels and nodes are depicted in green in the figure.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 8 1 Nasopharyngeal cancer basics Risk factors | Treatment types

There are three classes of nasopharyngeal , the cause of nasopharyngeal cancer for carcinoma: most people appears to be an interaction of the with other factors. In the United States, † Nonkeratinizing carcinoma the virus is a less common cause.

† Keratinizing

† Basaloid squamous cell carcinoma Treatment types Nasopharyngeal cancers are further grouped by how they look under a microscope. This section briefly describes treatments for nasopharyngeal cancer. Not everyone receives † Well differentiated cancers look like normal the same treatment. Your doctor will tailor tissue. treatment to you based on tests described in † Poorly differentiated cancers look less like in Part 2. Treatment options based on cancer normal tissue. features are listed in Part 3.

† Undifferentiated cancers don’t look like normal tissue. Clinical trial One treatment choice may be a clinical trial. Nonkeratinizing carcinomas can be Clinical trials are strongly supported by NCCN. undifferentiated. Among nasopharyngeal NCCN believes that you will receive the best cancers, the undifferentiated type is the most management if treated in a clinical trial. common. It is also called a . A clinical trial is a type of research that studies a promising test or treatment in people. It gives people access to health care that otherwise Risk factors couldn’t usually be received. Clinical trials of treatment often include new treatments added A risk factor is anything that increases your to standard treatments. Ask your treatment chance of cancer. Worldwide, several risk team if there is an open clinical trial that you factors are linked to nasopharyngeal cancer. can join.

† In some parts of the world, nasopharyngeal cancer is commonly linked to alcohol and tobacco use. Radiation therapy uses high-energy x-rays or particles to treat nasopharyngeal cancer. It † In other areas, it is linked to often eating damages cancer cells. The cancer cells either salt-cured foods. die or stop making new cancer cells. † There is a very strong link between nasopharyngeal cancer and the Epstein- External beam radiation therapy (EBRT) is Barr virus. used for treatment. A large machine makes radiation beams that are shaped to the form of Most people who have the Epstein-Barr virus the tumor. The highest radiation dose is aimed do not get nasopharyngeal cancer. In and at the cancer. A much lower dose is given to

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 9 1 Nasopharyngeal cancer basics Treatment types

nearby tissue. NCCN experts recommend the cells grow. Cetuximab is a targeted therapy following techniques: that stops growth signals from a structure called EGFR that is on cancer cells. It also † Intensity-modulated radiation therapy allows immune cells to destroy the cancer (IMRT) is the preferred technique. It cells. It is given with carboplatin to treat some delivers x-ray beams that very closely nasopharyngeal cancers. match the shape of the target and spares more normal tissue. Immunotherapy † Three-dimensional conformal radiation T cells are a type of white blood cell. They help therapy (3D-CRT) delivers an x-ray beam fight disease, including cancer. But, they are that matches the shape of the target but unable to attack cancer cells that have a protein may not be as focused as IMRT. called PD-L1.

† treats cancer with proton Immune checkpoint inhibitors are a type beams. Proton beams deliver radiation of immunotherapy. These drugs include mostly within the tumor. NCCN experts pembrolizumab and nivolumab. They allow advise to get proton therapy only when T cells to attack cancer cells with PD-L1. They x-ray beams would damage too much are routinely used to treat some advanced normal tissue. throat cancers but are also being tested for other uses. Chemotherapy is a treatment with drugs that Surgery stop the cell life cycle. As a result, cancer cells Surgery is a treatment that removes tumors or cannot make new cells. Chemotherapy can entire organs with cancer. It is not a common also cause cells to destroy themselves. treatment for tumors in the nasopharynx. More often, surgery that removes lymph nodes in the Cisplatin, carboplatin, 5-fluorouracil (5-FU), neck is done. This type of surgery is called a and gemcitabine are commonly used to treat neck dissection. nasopharyngeal cancer. There are other drugs that are less often used but can work.

Chemotherapy may be received with radiation therapy. It improves the results of radiation. When received at the same time, it is called chemoradiation. Chemotherapy may also be received alone. Chemotherapy by itself may be received first or last in a series of treatment.

Targeted therapy Targeted therapy is a treatment with drugs that stop some of the ways by which cancer

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 10 1 Nasopharyngeal cancer basics Review

Review

† The nasopharynx is the part of the throat that is behind the nose.

† Most nasopharyngeal cancers form from cells that line the surface of the upper throat.

† There is a strong link between nasopharyngeal cancer and the Epstein- Barr virus.

† Not everyone receives the same treatment for nasopharyngeal cancer. Your treatment will be partly based on test results.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 11 2 Testing for nasopharyngeal cancer

13 General health tests 14 Distress screening 15 Nasopharyngoscopy 16 Imaging 17 18 Epstein-Barr virus testing 18 Other tests 19 Review

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 12 2 Testing for nasopharyngeal cancer General health tests

Valid tests are needed to identify Guide 1. Health care before treatment nasopharyngeal cancer and plan treatment. This chapter describes tests Tests and services and other health care that you may receive before treatment. Medical history Physical exam including head and neck exam

Doctors plan treatment using many sources of Smoking treatment if needed information. These sources include the health Distress screening care listed in Guide 1. Another source is you. Tell your doctor your concerns and goals for Nasopharyngoscopy treatment. Together, you can share in the decision-making process. Read Part 4 to learn MRI with contrast from skull base to collarbone; your doctor may also order CT with contrast of skull more about making treatment decisions. base and neck

FDG PET/CT, chest CT with contrast, or both General health tests Biopsy Your doctor may order Epstein-Barr virus testing Medical history Your doctor will want to know about any health Speech or swallowing exam if needed problems and their treatment during your Nutrition assessment if needed lifetime. Be prepared to talk about: Dental exam and x-rays if needed † Illnesses Hearing or vision tests if needed † Injuries

† Health conditions Hormone tests if needed

† Symptoms

† Medications

† Tobacco use (now and in the past) Medications If you use tobacco products, it is important to quit. They can limit how well cancer treatment It may help to bring a list of works. Ask your treatment team about old and new medicines to your counseling and medicines that can help you doctor’s office. quit.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 13 2 Testing for nasopharyngeal cancer Distress screening

Family history Distress screening Some cancers and other health problems can run in families. Thus, your doctor will ask Distress is an unpleasant experience of a about the medical history of your close blood mental, physical, social, or spiritual nature. It relatives. Such family includes your siblings, can affect how you feel, think, and act. It can parents, and grandparents. Be prepared to tell include feelings of: who has had what diseases and at what ages. † Sadness

† Fear Physical exam A physical exam is a study of your body. It is † Helplessness done to look for signs of disease. It is also used † Worry to help assess what treatments may be options. † Anger During this exam, expect the following to be † Guilt checked:

† Your body temperature Everyone with cancer has some distress at some point in time. It is to be expected. † Your blood pressure

† Your pulse and breathing rate Feeling distressed may be a minor problem or it may be more serious. You may be so † Your weight distressed that you can’t do the things you used † How your lungs, heart, and gut sound to do. People with head and neck cancer are at risk for depression. † How your eyes, skin, nose, ears, and mouth look It is important that your treatment team knows † The size or hardness of your organs how you feel. They may ask you to complete a list of screening questions to assess how † Level of pain when you are touched distressed you are.

Head and neck exam If needed, your treatment team can get you An exam of your head and neck is key to help. Help can include support groups, talk planning treatment. Your doctor will carefully therapy, or medication. Some people also feel inspect your throat, mouth, nose, and ears. better by exercising, talking with loved ones, or Your doctor will feel for lumps under your jaw using relaxation techniques. There may also be and along your neck. helpful community resources, such as support groups and wellness centers. To look inside your head, your doctor may use a device. One type of device is a small mirror like what your dentist uses to look inside your mouth. Your doctor may also examine your throat with a fiberoptic endoscope.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 14 2 Testing for nasopharyngeal cancer Nasopharyngoscopy

Visit this webpage to read more about Nasopharyngoscopy distress related to cancer: NCCN.org/patients/ guidelines/cancers.aspx#distress. Nasopharyngoscopy is a procedure that allows doctors to see the upper throat. Your doctor will

our Pleaseonline completesurvey at NCCN.org/patients/survey use a hand-held device called an endoscope.

NCCN GUIDELINES Only the thin, tube-shaped part of the device is FOR PATIENTS® Version 1.2017 guided through your nose and into your throat.

Distress At the tip of the scope is a light and tiny

SUPPORTIVE CARE BOOK SERIES camera. Your doctor will assess the tumor and Presented with support from: may remove a tissue sample. Sometimes, the sample can be removed in the clinic, but sometimes, it must be done in an operating room. See Figure 3.

Available online at NCCN.org/patients

Figure 3 Nasopharyngoscopy

A nasopharyngoscopy is a procedure that allows doctors to see the upper throat. Your doctor will use a hand-held device called an endoscope.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 15 2 Testing for nasopharyngeal cancer Imaging

Imaging Skull base The skull is a group of bones in your head Imaging makes pictures of the insides of your that protects your brain. The base of the body. It is used to detect cancer in deep tissue, skull is between the bottom of your brain and lymph nodes, or distant body parts. Some the structures of your face. It is common for imaging also reveals some features of a tumor nasopharyngeal cancer to grow into the skull and its cells. base or very near to it.

A radiologist is a doctor who’s an expert in Your doctor will order magnetic resonance reading images. This doctor will convey the test imaging (MRI) with contrast. The MRI machine results to your other doctors. makes 3-D images with a magnetic field and radio waves. The imaging will include the base Some imaging tests use contrast. It is a of your skull down to your collarbone. substance that is often injected into the bloodstream. It makes the images easier to Your doctor may also order computed read. Tell your doctor if you’ve had problems tomography (CT) of your skull base and with contrast in the past. Also, allergies to neck. The CT machine makes 3-D images shellfish may mean you’ll be allergic to some by combining many x-rays taken at different types of contrast. angles. See Figure 4.

Figure 4 CT machine

Pictures of the insides of your body can be made with imaging. During the scan, you will lie on a table that will move into the tunnel of the machine. The pictures will be viewed by a doctor who will look for signs of cancer.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 16 2 Testing for nasopharyngeal cancer Biopsy

Distant cancer spread Nasopharyngeal cancer can spread to distant body parts. Distant spread at the time of diagnosis is common. It can be seen with whole-body PET/CT, chest CT, or both.

PET stands for positron emission tomography. It requires injection of a small Team work amount of radiotracer into your bloodstream. Fluorodeoxyglucose (FDG) is the radiotracer that is almost always used. It helps to detect It takes a team of health care providers to treat even small amounts of cancer. nasopharyngeal cancer. Your treatment team may include a:

Biopsy 3 Radiation oncologist 3 Medical oncologist A biopsy is a procedure that removes tissue 3 Head and neck surgeon or fluid samples for testing. Doctors use the results of the physical exam and imaging 3 Nurse to decide where to do a biopsy. In a lab, 3 Pathologist a pathologist will study the samples with 3 Radiologist a microscope. The lab tests will conclude whether you have cancer. 3 Oncologic dentist 3 Eye doctor Your doctor will decide which type of biopsy 3 Ear doctor is needed. 3 Plastic surgeon † Your doctor may use a scissor-like tool 3 Speech therapist to remove part or all of the tumor. 3 Physical therapist † A fine-needle aspiration (FNA) removes a small number of cells and is preferred 3 Registered dietician for a neck lump. 3 Case manager

† A core needle biopsy removes a small 3 Mental health professional piece of tissue with a hollow needle. 3 Supportive care specialist All biopsy results are included in a pathology report. This report will be sent to your doctor. Ask for a copy and a meeting to discuss the results. Take notes and ask questions.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 17 2 Testing for nasopharyngeal cancer Epstein-Barr virus testing | Other tests

Epstein-Barr virus testing You will be given a therapy plan to reduce problems. Not everyone with Epstein-Barr virus gets nasopharyngeal cancer. But, there is a very Nutrition assessment strong link between the virus and the cancer. Some throat cancers make it hard to eat. It may Your doctor may decide to test for the virus be painful to chew or swallow. In this case, you if the cancer class is nonkeratinizing or the may see a registered dietician before cancer cancer looks undifferentiated. A blood or tumor treatment. Be prepared to report what you sample can be tested to learn if the Epstein- eat and drink. Based on your response, the Barr virus is in the tumor. dietician will make a nutritional plan for you.

You may be assessed for a feeding tube. There are different types. A nasogastric tube is Other tests placed through the nose, down the throat, and into the stomach. A percutaneous endoscopic There are many key body parts in the head and gastrostomy (PEG) tube is placed through the neck. Nasopharyngeal cancer or its treatment skin directly into the stomach. can cause problems with one or more of them. Based on your symptoms and cancer tests, you may get one or more of these tests: Dental exam If needed, a dental exam can help your cancer † Speech and swallowing exam doctor plan treatment. It may also prevent † Nutrition assessment health problems caused by treatment. When fewer problems occur, cancer treatments may † Dental exam work better. Ask your cancer center for a list of † Hearing test local dentists who can help.

† Eye exam During a dental exam, the dentist will inspect † Hormone tests your mouth and teeth. X-rays of your teeth will also be done. Cavities will be fixed. Unhealthy teeth will be removed to prevent problems Speech and swallowing exam during treatment. Diseases of the mouth will Throat cancer or its treatment can cause also be treated. problems with speech or swallowing. If you have or will have problems, you should see a Your dentist will teach you how to take care of speech-language pathologist. Before cancer your teeth. Proper brushing, flossing, and diet treatment, this expert will assess how well you are needed. Before cancer treatment, custom- speak and swallow. made trays for fluoride treatment may be made. The speech-language pathologist will watch Cancer treatments may cause dental problems. and listen as you talk and eat. You may also get Your dentist will teach you about these health a swallow test called a video fluoroscopic study.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 18 2 Testing for nasopharyngeal cancer Review problems. You will also learn how prevent or Review stop such problems. † A medical history is a report of all health events and treatment in your lifetime. Hearing test Expect to be asked questions about your Nasopharyngeal cancer or its treatment can health and the health of some family affect the ears. The cancer can grow and members. If you smoke, your treatment press on the inner ear called the eustachian team can get you help to quit. tube. Cancer symptoms include frequent ear , , ear pain, and ringing in † A physical exam is a study of your body. the ear. If needed, your cancer doctor may refer During a head and neck exam, your doctor you to get a hearing test. will carefully inspect your throat, mouth, nose, and ears.

Eye exam † People with head and neck cancer are at Nasopharyngeal cancer or its treatment can risk for depression. Your treatment team affect the eyes. Symptoms of the cancer can get you help. include blurred or double vision. If needed, your † Your doctor will examine your upper throat cancer doctor will refer you to an eye doctor by inserting part of a scope through your (ophthalmologist). An eye exam includes tests nose. of your vision and checks the health of your eyes. † Imaging allows your doctors to see inside your body without cutting into it. It is used to assess the skull base and distant body Hormone tests parts for cancer. Endocrine glands are organs that make hormones. The pituitary gland is in the brain. It † A biopsy removes a small piece of tissue is often called the “master gland” of the body. for testing. It is needed to learn if you have The hypothalamus is a region of the brain that cancer. controls the pituitary gland. † Your doctor may want to test for the Epstein-Barr virus. The decision to Some nasopharyngeal tumors grow close to the test partly depends on the type of pituitary gland and hypothalamus. Cancer or nasopharyngeal cancer. its treatment can affect these brain structures. In turn, fertility, metabolism, and other body † Nasopharyngeal cancer and its treatment functions are altered. may cause problems with key body parts in your head and neck. Your cancer doctor Your cancer doctor may refer you to an may refer you for tests given by other endocrinologist. This doctor is an expert in specialists. glands and hormones. The level of several hormones in your body may be measured.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 19 3 Treating nasopharyngeal cancer

21 Overview 23 Initial treatment 26 Neck dissection 28 Follow-up care 30 Recurrent or persistent cancer 32 Review

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 20 3 Treating nasopharyngeal cancer Overview

Treatment of nasopharyngeal cancer Many nasopharyngeal cancers are cured. If a is based on many factors. Read this cure is not possible, the goals of treatment are chapter to learn the options for each to reduce symptoms, control cancer growth, point of care. And, discuss with your and extend life. During and after cancer doctor which of these options are right treatment, you may meet with health providers for you. whose focus is to improve your quality of life.

Initial treatment Your doctor will plan your initial treatment based Overview on many factors. These factors include your age and level of fitness. Treatment will also be Treatment of nasopharyngeal cancer includes based on the cancer. A very important factor is treatment of the cancer and support for you. the cancer stage. See Guide 2.

Guide 2. Nasopharyngeal cancer stages

Stage Description

• There is a tumor in the nasopharynx only, or Stage 1 • There is a tumor in the nasopharynx and it has invaded the middle of the throat or the nose

• The cancer has spread to lymph nodes on one side of the neck and these lymph nodes are 6 centimeters or smaller in size, or • The cancer has spread to lymph nodes behind the throat and these lymph nodes are Stage 2 6 centimeters or smaller in size, or • There is a tumor in the nasopharynx and it has invaded the space between the skull base and lower jaw (parapharyngeal space) or nearby muscles

• The cancer has spread to lymph nodes on both sides of the neck and these lymph nodes are 6 centimeters or smaller in size, or Stage 3 • There is a tumor in the nasopharynx and it has invaded 1) the bones below the skull or in the neck or 2) nearby sinuses

• The cancer has spread to lymph nodes on both sides of the neck and these lymph nodes are larger than 6 centimeters, or • The cancer has spread to lymph nodes on both sides of the neck and these lymph nodes Stage 4A are in the lowest part of the neck, or • There is a tumor in the nasopharynx and it has invaded the skull, cranial nerves, lower throat, bone around the eye, main saliva gland, or soft tissues of the jaw

Stage 4B • The cancer has spread to body parts far from the nasopharynx

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 21 3 Treating nasopharyngeal cancer Overview

Cancer stage sometimes given after primary treatment to The cancer stage describes the extent stop the cancer from returning. For some of cancer in your body. Nasopharyngeal cancers, induction chemotherapy is the first cancer almost always forms a tumor in the treatment given. It may improve outcomes by nasopharynx. The tumor may invade nearby treating cancer throughout the body early in the tissue, such as the skull. treatment process.

Nasopharyngeal cancer often spreads to other Neck dissection body parts. It often spreads to lymph nodes Nasopharyngeal cancer is very likely to spread behind the ears and in the neck. It is also to lymph nodes in the neck. Surgery to remove likely to spread to distant body parts like the lymph nodes that may have cancer is called a lungs, liver, bones, and lymph nodes below the neck dissection. In this chapter, who will likely collarbone. need a neck dissection is explained. Nasopharyngeal cancer has six stages based on its extent. Follow-up care You may start follow-up care when there are no † Stage 0 isn’t cancer but abnormal throat signs of cancer. During follow-up care, you will cells that may become cancer. have scheduled visits with your cancer doctor. † Stages 1, 2, 3, 4A, and 4B are cancer. You will receive tests to assess the cancer has returned. Initial treatment approach Radiation therapy is often used for initial Recurrent or persistent cancer treatment. It works very well for many The last section in this chapter is about nasopharyngeal cancers. It may be given treatment for recurrent or perisistent cancer. during the same time period as chemotherapy. In this book, this combined treatment is † Recurrent cancer is cancer that returns called “chemoradiation.” Some people after a cancer-free time period. call it “concurrent radiation therapy and † Persistent cancer is cancer that remains in chemotherapy.” the body after treatment. For cancers that have spread far, cancer drugs Recurrent and persistent cancers are treated are commonly used for treatment. The drugs alike. Systemic therapy is commonly used. travel in the bloodstream to where the cancer is. Another name for this type of treatment is Like initial treatment, your options will partly systemic therapy. Types of systemic therapy depend on the extent of the cancer. Also, what are chemotherapy, targeted therapy, and you have already received for treatment affects immunotherapy. your options. Some treatments can only be given if certain markers are in the cancer cells. Initial treatment may consist of a series of treatments. Primary treatment is the main treatment of cancer. Adjuvant treatment is

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 22 3 Treating nasopharyngeal cancer Initial treatment

Initial treatment Stage 1 The initial treatment for stage 1 is radiation Options for initial treatment are mainly based therapy. Other types of treatment are not on the cancer stage. Ask your doctor what needed because the cancer is small. the goals of your treatment are and what you † The cancer in your nasopharynx will be should expect during treatment. Treatment treated with high-dose radiation. options by cancer stage are listed in Guide 3 and are described next. † Your neck will be treated with low-dose radiation in case there are cancer cells in lymph nodes.

Guide 3. Options for initial treatment There may be so few cancer cells in lymph nodes that they weren’t found by imaging or biopsy. Radiation can treat these cancer cells in Stage 1 your neck.

• Radiation therapy to the nasopharynx; the Stages 2, 3, and 4A neck may be treated, too Compared to stage 1, more intense treatment is needed for stages 2, 3, and 4A. Often, more than one type of treatment is used. It is very Stages 2, 3, and 4A common to be treated with both radiation therapy and chemotherapy. • Clinical trial (preferred) Your nasopharynx will be treated with higher • Chemoradiation with chemotherapy doses of radiation. High-dose radiation will • Chemoradiation also be used to treat parts of your neck that have more cancer. Chemotherapy increases the effectiveness of the radiation. Treatment Stage 4B options include:

• Clinical trial (preferred) Clinical trial • Platinum-based systemic therapy followed by: NCCN experts prefer a clinical trial for stage 2, ◦ Radiation therapy or 3, and 4A cancers. More research is needed to ◦ Chemoradiation or learn which treatment is best. Ask your doctor if ◦ No further treatment there is a clinical trial that is right for you. • Chemoradiation • Radiation therapy or surgery Chemoradiation with chemotherapy For stages 2, 3, and 4A, chemoradiation achieves better results than radiation therapy alone. The combined treatment extends life. It also helps prevent the cancer from returning.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 23 3 Treating nasopharyngeal cancer Initial treatment

Adding chemotherapy to chemoradiation may improve outcomes for some people. You IN DEPTH may receive chemotherapy before or after chemoradiation. Induction chemotherapy is Chemoradiation received before chemoradiation. Adjuvant chemotherapy is received afterward. with chemotherapy

Chemoradiation For stages 2, 3, and 4A, NCCN experts Adding chemotherapy to chemoradiation may include chemoradiation alone as an improve results. Induction chemotherapy is option. Cisplatin is the chemotherapy drug received before chemoradiation. Adjuvant most often used with radiation therapy. chemotherapy is received afterward. The Chemoradiation alone may be an option for following regimens are options. advanced stages when the cancer is limited. There are ongoing studies that will help Induction chemotherapy and define who can have chemoradiation alone chemoradiation and who needs more treatment. Common induction regimens • Docetaxel, cisplatin, and 5-FU Stage 4B • Cisplatin and 5-FU Stage 4B is metastatic cancer. The cancer has spread to distant body parts. Metastatic • Cisplatin, epirubicin, and paclitaxel cancer can be grouped by limited versus • Docetaxel and cisplatin widespread disease. Treatment options may Common chemoradiation regimens differ based on the extent of the . • Cisplatin or carboplatin is often used for chemoradiation that is received after Clinical trial induction chemotherapy NCCN experts prefer a clinical trial for stage 4B cancers. New treatments are being studied. Ask your doctor if there is a clinical Chemoradiation and adjuvant trial that is right for you. chemotherapy Common chemoradiation regimens Platinum-based systemic therapy • Cisplatin is often used for chemoradiation For widespread metastatic cancer, systemic therapy is a common treatment. Cisplatin or when it is the first treatment received carboplatin—drugs made with platinum— Common adjuvant regimens combined with another cancer drug is • Cisplatin and 5-FU standard treatment. Combined treatment may • Carboplatin and 5-FU be too toxic, so you may receive one cancer drug instead.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 24 3 Treating nasopharyngeal cancer Initial treatment

After systemic therapy, you may receive more treatment to the throat or neck. This extra IN DEPTH treatment may help reduce symptoms caused by the cancer. Radiation therapy or Primary systemic therapy chemoradiation may be used.

Chemoradiation Radiation therapy and surgery may not be Chemoradiation may be used for treatment if treatment options. This is often the case for the distant disease is limited. It can also be metastatic and recurrent cancers. Instead, used to relieve cancer symptoms caused by a systemic therapy may be used. Types of systemic tumor in the throat or lymph nodes. therapy are chemotherapy, targeted therapy, and Radiation therapy or surgery immunotherapy. Distant disease may consist of only one or a few tumors. Examples include one tumor in the First-line regimens lung or bone. In these cases, radiation therapy Combination regimens or surgery may be used for treatment. • Cisplatin and gemcitabine (preferred) • Cisplatin and 5-FU • (Cisplatin or carboplatin) and docetaxel • (Cisplatin or carboplatin) and paclitaxel • Carboplatin and cetuximab Single agents • Cisplatin • Carboplatin • Paclitaxel • Docetaxel • 5-FU • Methotrexate • Gemcitabine • Capecitabine

Second-line regimens

• First-line regimens • Nivolumab for recurrent or metastatic non- keratinizing cancer • Pembrolizumab for recurrent or metastatic PD-L1–positive cancer

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 25 3 Treating nasopharyngeal cancer Neck dissection

Neck dissection

A neck dissection is a surgery that removes lymph nodes that likely have cancer. It may help stop a cancer recurrence in the neck for some people. This section explains who will likely benefit from a neck dissection. Supportive care

Cancer tests Supportive care aims to improve your quality of life. After initial treatment, your doctor will assess It includes care for health issues caused by cancer the treatment results. It may takes weeks for or cancer treatment. It is also sometimes called the true results to be seen. So, your doctor may wait 4 to 8 weeks after treatment to do a clinical palliative care. Supportive care is important for assessment. everyone, not just people at the end of life.

A clinical assessment may include: Treatment side effects † Questions about symptoms All cancer treatments can cause unwanted health † A physical exam issues. Such health issues are called side effects. † An endoscopy of your throat Some side effects may be harmful to your health. Others may just be unpleasant. Some people get imaging of the neck after the clinical assessment. Your doctor will likely order imaging if there are no signs of cancer. If Side effects depend on many factors. These there are signs of cancer, imaging may not be factors include the treatment type, length or dose of needed. The types and timing of imaging after treatment, and the person. cancer treatment are listed in Guide 4.

Ask your treatment team for a complete list of side Who gets a neck dissection? effects of your treatments. Also, tell your treatment Your doctor will decide whether a neck dissection is needed based on test results. For team about any new or worse symptoms you get. most people, there is no cancer left after initial There may be ways to help you feel better. There treatment, so surgery is not often needed. See are also ways to prevent some side effects. Guide 5 for a summary of who will need a neck dissection.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 26 3 Treating nasopharyngeal cancer Neck dissection

Guide 4. Imaging after initial treatment

Results of clinical assessment What are the options?

• FDG PET/CT at least 12 weeks after treatment ◦ If cancer is detected with FDG PET/CT, get CT with Your doctor did not find signs of cancer contrast of your throat and neck, MRI with contrast, or both • CT with contrast of your throat and neck, MRI with contrast, or both to be done 8 to 12 weeks after treatment has ended

• Imaging may not be done

• CT with contrast of your throat and neck, MRI with contrast, Your doctor did find signs of cancer or both to be done 4 to 8 weeks after treatment has ended

• FDG PET/CT

Guide 5. Neck dissection

Test results What are the options?

Your doctor did not find signs of cancer on clinical • A neck dissection is not needed; start follow-up care assessment and there were no signs on imaging

Your doctor did not find signs of cancer on clinical • You may need a neck dissection but your doctor assessment but CT or MRI showed signs of may first order FDG-PET to help guide treatment cancer decisions

• You may have a choice. Talk with your doctor Your doctor did not find signs of cancer on clinical about starting follow-up care or getting a neck assessment but PET/CT showed signs of cancer dissection. An ultrasound-guided FNA may help or lymph nodes are larger than 1 centimeter guide your decision.

Your doctor did not find signs of cancer on clinical assessment but PET/CT showed signs of cancer • A neck dissection is needed and lymph nodes are larger than 1 centimeter

Your doctor found signs of cancer by clinical • You may get surgery to remove the , assessment a neck dissection, or both

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 27 3 Treating nasopharyngeal cancer Follow-up care

Follow-up care Six years after treatment, you may see your doctor only once a year. See Guide 6 for a Follow-up care is important for your long-term schedule of visits. health. It is a part of survivorship care. It is started when there are no signs of cancer. Imaging Imaging may be done to assess for relapse. Many types of health care providers may be † Within 6 months after treatment has involved in your follow-up care. Examples ended, your doctor may repeat the include your primary care doctor, cancer doctor, imaging that was done before treatment. and other specialists. Your providers should work together to meet your needs. † Your doctor may order imaging on a regular basis if some parts of your neck or Your cancer doctor will provide you with a head can’t be seen on exam. survivorship care plan. This plan contains key † If signs of cancer appear, imaging will be information for your long-term health, such as: needed at that time. † A list of your cancer treatments and supportive care that you have had Epstein-Barr virus Some cancer centers test for DNA of the † Tests to detect a recurrence Epstein-Barr virus during follow-up care. A † A list of side effects that you may get and blood sample is used for testing. Testing may ways to prevent or treat them help find a recurrence, but more research is needed to prove how helpful this test is. † Screening for other cancers

† Health care to prevent disease Guide 6. Follow-up visits † Goals and help for a healthy lifestyle Year after How often are visits needed? Tests for recurrence treatment Follow-up care should include a schedule of Year 1 • Every 1 to 3 months tests for relapse. Routine testing for cancer recurrence is called surveillance. Early Year 2 • Every 2 to 6 months detection will allow for timely treatment. Year 3 to 5 • Every 4 to 8 months

Follow-up visits Year 6 and on • Every 12 months You will meet with your cancer doctor often after treatment ends. At these visits, your doctor may perform the following:

† Medical history

† Physical exam

† Endoscopy of your throat

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 28 3 Treating nasopharyngeal cancer Follow-up care

Long-term and late side effects Cancer screening Some side effects appear shortly after cancer You may be at risk for a second cancer. A treatment starts and quickly resolve after second cancer is a possible late effect of some treatment ends. Other side effects are long- cancer treatments. Also, your risk for cancer term or may appear years later. increases as you age and if you smoke, the longer you do so. Ask your doctor about your At follow-up visits, your doctor will assess for risk for another cancer. side effects. Tell your doctor about any new or worse symptoms. Side effects that may occur At follow-up visits, your doctor will examine after treatment include: your body for signs of cancer. You may also get a blood test to look for signs of cancer. † Vision problems If you have a high risk for certain cancers, † Hearing problems you may enroll in a screening program. † Swallowing problems Cancer screening is routine testing for cancer before cancer symptoms start. There is not a † Tiredness screening program for every type of cancer. † Swelling There are cancer screening programs for: † Dry nose and mouth † Prostate cancer in men † Pain † Breast or in women Examples of ways to prevent or treat side † Colorectal cancer effects are: † † You may receive thyroid hormone replacement for thyroid problems. People at high risk for lung cancer should † You may receive treatment for ear enroll in a screening program. Visit this infections or pain. webpage to read about lung cancer screening: nccn.org/patients/guidelines/cancers. † A dentist can help you cope with dry aspx#lungScreening. mouth.

† A speech-language pathologist can Disease prevention improve your speech or swallowing. Another part of survivorship care is to prevent † A dietician can help you get good nutrition. diseases. Such care can include getting immunization shots for the flu, herpes, shingles, † Mental health providers can help you quit and other diseases. Dental cleaning and exams smoking and limit alcohol. on a regular basis can prevent disease, too. † Mental health providers can also treat depression and anxiety.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 29 3 Treating nasopharyngeal cancer Recurrent or persistent cancer

Healthy lifestyle Local treatment It’s important to start or keep a healthy lifestyle. Local treatment includes surgery, radiation Healthy living may improve your health and therapy, and chemoradiation. It is more wellbeing. It may also help prevent the cancer often used to treat locoregional cancer than from returning. Work with your treatment team metastatic cancer. Metastatic cancer must be to set goals and make plans for healthy living. within a limited area for local treatment to be an option. Common goals for healthy living include: Radiation therapy may be an option if you had † Seeing a primary care provider on a it before. There are limits to how much radiation regular basis a body part can safely receive. NCCN experts † Limiting alcohol use prefer that a second course of radiation therapy be received within a clinical trial. † Not using tobacco products

† Using sun protection Systemic therapy † Maintaining a healthy weight Systemic therapy by itself is often used when local treatment is not an option. If you are † Eating a balanced diet healthy enough, regimens may contain more † Drinking enough fluids than one cancer drug. If your health is poor, your doctor may prescribe one cancer drug for † Exercising your safety.

Most options for systemic therapy consist of chemotherapy only. One option includes Recurrent or persistent cancer targeted therapy—carboplatin with cetuximab. Immunotherapy (nivolumab or pembrolizumab) Cancer may return or persist but not spread far. is an option, too. This is called locoregional cancer. Cancer that has spread far from the nasopharynx is called metastatic disease. Options for recurrent or Clinical trial persistent cancer based on its extent are listed A clinical trial may be an option. Ask your doctor in Guide 7 and described in this section. if there’s a clinical trial that is right for you. A clinical trial may test which current treatment is Your treatment options will be based on many best or may test a new treatment. factors. One factor is whether the cancer can be fully and safely removed by surgery. Supportive care Your options may also depend on if you had Supportive care aims to improve your quality of radiation therapy in the past. life. The goal is to prevent or relieve symptoms instead of cure or control the cancer. Radiation therapy may be used to relieve symptoms.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 30 3 Treating nasopharyngeal cancer Recurrent or persistent cancer

Guide 7. Options for recurrent or persistent cancer The cancer has not spread far (locoregional)

Treatment status What are the options?

• Surgery followed by one of the following: The cancer can be removed by surgery ◦ No further treatment if all the cancer was likely removed and you have had radiation therapy or if the area can’t be treated with radiation again ◦ Radiation therapy or chemoradiation; receiving treatment within a clinical trial is preferred

• Surgery followed by one of the following: ◦ No further treatment if all the cancer was likely removed The cancer can be removed by surgery ◦ Chemoradiation and you haven’t had radiation therapy ◦ Radiation therapy • Chemoradiation

• Radiation therapy or chemoradiation; receiving treatment The cancer can’t be removed by surgery within a clinical trial is preferred and you have had radiation therapy • Systemic therapy • Supportive care

• Clinical trial (preferred) • Chemoradiation • Induction chemotherapy followed by either radiation therapy The cancer can’t be removed by surgery or chemoradiation and you haven’t had radiation therapy • Radiation therapy • Single-agent systemic therapy • Supportive care including radiation therapy

The cancer has spread far (metastatic)

What are the options?

• Clinical trial (preferred) • Systemic therapy • Surgery, radiation therapy, or chemoradiation for limited distant spread • Supportive care

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 31 3 Treating nasopharyngeal cancer Review

Review

† The goal of treatment is to either cure the cancer or stop the cancer from growing.

† Initial treatment is mainly based on the cancer stage. A clinical trial is the preferred treatment option for cancer stages 2, 3, and 4. Radiation, chemotherapy, or both work well for many nasopharyngeal cancers.

† You may undergo a neck dissection if cancer remains in your neck lymph nodes after initial treatment.

† Get a survivorship care plan from your cancer doctor. This plan should include a schedule of follow-up visits with your cancer doctor. It should also list health care to prevent other diseases.

† Nasopharyngeal cancer may return or persist after initial treatment. Options mainly depend on the extent of the cancer and your prior treatment.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 32 4 Making treatment decisions

34 It’s your choice 34 Questions to ask your doctors 39 Deciding between options 40 Websites 40 Review

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 33 4 Making treatment decisions It’s your choice | Questions to ask

Having cancer is very stressful. There together, you are likely to get a higher quality of is a lot to learn in what feels like a short care and be more satisfied. You’ll likely get the amount of time. This chapter can help treatment you want, at the place you want, and you make decisions that reflect your by the doctors you want. beliefs, wishes, and values.

Questions to ask your doctors It’s your choice You may meet with experts from different fields of medicine. Strive to have helpful talks with The role patients want in choosing their each person. Prepare questions before your treatment differs. You may feel uneasy about visit and ask questions if the person isn’t clear. making treatment decisions. This may be due You can also take notes and get copies of your to a high level of stress. It may be hard to hear medical records. or know what others are saying. Stress, pain, and drugs can limit your ability to make good It may be helpful to have your spouse, partner, decisions. You may feel uneasy because you family member, or a friend with you at these don’t know much about cancer. You’ve never visits. A patient advocate or navigator might heard the words used to describe cancer, tests, also be able to come. They can help to ask or treatments. Likewise, you may think that your questions and remember what was said. judgment isn’t any better than your doctors’. Suggested questions to ask are listed on the following pages. Letting others decide which option is best may make you feel more at ease. But, whom do you want to make the decisions? You may rely on your doctors alone to make the right decisions. However, your doctors may not tell you which to choose if you have more than one good option. You can also have loved ones help. They can gather information, speak on your behalf, and share in decision-making with your doctors. Even if others decide which treatment you will receive, you still have to agree by signing a consent form.

On the other hand, you may want to take the lead or share in decision-making. Most patients do. In shared decision-making, you and your doctors share information, weigh the options, and agree on a treatment plan. Your doctors know the science behind your plan but you know your concerns and goals. By working

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 34 4 Making treatment decisions Questions to ask your doctors

What’s my diagnosis and prognosis?

It’s important to know that there are different types of cancer. Cancers with the same name can even greatly differ. Based on your test results, your doctor can tell you which type of cancer you have. Some people want to know the prognosis. A prognosis is a prediction of the pattern and outcome of a disease. Knowing the prognosis may affect what you decide about treatment.

1. What type of cancer do I have? From what type of cell did it form? Is this cancer common?

2. What is the cancer stage? Does this stage mean the cancer is advanced?

3. Is this a fast- or slow-growing cancer?

4. What tests do you recommend for me?

5. Where will the tests take place? How long will the tests take and will any test hurt?

6. What if I am pregnant?

7. How do I prepare for testing?

8. Should I bring a list of my medications?

9. Should I bring someone with me?

10. How often are these tests wrong?

11. Would you give me a copy of the pathology report and other test results?

12. Who will talk with me about the next steps? When?

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 35 4 Making treatment decisions Questions to ask your doctors

What are my options?

There is no single treatment practice that is best for all people. There is often more than one treatment option along with clinical trial options. Your doctor will review your test results and recommend treatment options.

1. What will happen if I do nothing?

2. Can I just carefully monitor the cancer?

3. Do you consult NCCN recommendations when considering options?

4. Are you suggesting options other than what NCCN recommends? If yes, why?

5. Do your suggested options include clinical trials? Please explain why.

6. How do my age, health, and other factors affect my options? What if I am pregnant?

7. Which option is proven to work best?

8. Which options lack scientific proof?

9. What are the benefits of each option? Does any option offer a cure or long-term cancer control? Are my chances any better for one option than another? Less time-consuming? Less expensive?

10. What are the risks of each option? What are possible complications? What are the rare and common side effects? Short-lived and long-lasting side effects? Serious or mild side effects? Other risks?

11. How will treatment affect my looks, speech, chewing, and swallowing? Will my sense of smell or taste change?

12. How do you know if treatment is working?

13. What are my options if treatment doesn’t work?

14. What can be done to prevent or relieve the side effects of treatment?

15. What are my chances that the cancer will return?

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 36 4 Making treatment decisions Questions to ask your doctors

What does each option require of me?

Many patients consider how each option will practically affect their lives.This information may be important because you have family, jobs, and other duties to take care of. You also may be concerned about getting the help you need. If you have more than one option, choosing the option that is the least taxing may be important to you:

1. Will I have to go to the hospital or elsewhere? How often? How long is each visit?

2. What do I need to think about if I will travel for treatment?

3. Do I have a choice of when to begin treatment? Can I choose the days and times of treatment?

4. How do I prepare for treatment? Do I have to stop taking any of my medicines? Are there foods I will have to avoid?

5. Should I bring someone with me when I get treated?

6. Will the treatment hurt?

7. How much will the treatment cost me? What does my insurance cover?

8. Will I miss work or school? Will I be able to drive?

9. Is home care after treatment needed? If yes, what type?

10. How soon will I be able to manage my own health?

11. When will I be able to return to my normal activities?

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 37 4 Making treatment decisions Questions to ask your doctors

What is your experience?

Research suggests that patients treated by more experienced doctors have better results. It is important to learn if doctors are experts in the care that they offer.

1. Are you board-certified? If yes, in what area?

2. How many patients like me have you treated?

3. How many procedures like the one you’re suggesting have you done?

4. Is this treatment a major part of your practice?

5. How many of your patients have had complications?

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 38 4 Making treatment decisions Deciding between options

Deciding between options Support groups In addition to speaking with health experts, it Deciding which option is best can be hard. may help to talk to patients who have walked Doctors from different fields of medicine in your shoes. Support groups often consist of may have different opinions on which option people at different stages of treatment. Some is best for you. This can be very confusing. may be in the process of deciding while others Your spouse, partner, friends, or family may may be finished with treatment. At support disagree with which option you want. This can groups, you can ask questions and hear about be stressful. In some cases, one option hasn’t the experiences of other people with throat been shown to work better than another. Some cancer. ways to decide on treatment are discussed next. Compare benefits and downsides Every option has benefits and downsides. Second opinion Consider these when deciding which option is After finding out you have cancer, it is normal best for you. Talking to others can help identify to want to start treatment as soon as possible. benefits and downsides you haven’t thought of. While cancer can’t be ignored, there is time to For example, you can decide how aggressive have another doctor review your test results you want to be with treatment at the cost of and suggest a treatment plan. This is called increasing negative long-term side effects. getting a second opinion, and it’s a normal part Scoring each factor from 0 to 10 can also help of cancer care. since some factors may be more important to you than others. You should feel comfortable Getting a second opinion doesn’t mean you discussing your goals of care with your health don’t trust the first doctor. In fact, most doctors care team. who are diagnosed with cancer will see more than one doctor before beginning treatment. What’s more, some health plans require a second opinion. If your health plan doesn’t cover the cost of a second opinion, you have the choice of paying for it yourself.

If the two opinions are the same, you may feel better about the treatment you accept to have. If the two opinions differ, think about getting a third opinion. Choosing your cancer treatment is a very important decision. It can affect your length and quality of life.

On the other hand, you might not feel a need for a second opinion. For some people, a second opinion causes more delay, anxiety, and stress.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 39 4 Making treatment decisions Websites | Review

Websites Review

† Shared decision-making is a process in American Cancer Society which you and your doctors plan treatment cancer.org/cancer/nasopharyngeal-cancer.html together.

† Asking your doctors questions is vital to Head and Neck Cancer Alliance (HNCA) getting the information you need to make headandneck.org informed decisions.

† Getting a second opinion, attending National Cancer Institute (NCI) support groups, and comparing benefits cancer.gov/types/head-and-neck and risks may help you decide which treatment is best for you. NCCN for Patients® nccn.org/patients

Oral Cancer Foundation oralcancerfoundation.org

smokefree.gov smokefree.gov

Support for People with Oral and Head and Neck Cancer (SPOHNC) spohnc.org

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 40 Ü Words to know

distress Words to know An unpleasant experience of a mental, physical, social, or spiritual nature.

3D-CRT EBRT Three-dimensional conformal radiation therapy External beam radiation therapy 5-FU endoscope 5-fluorouracil A device that is passed through a natural opening to do work inside the body. adjuvant therapy Treatment that is given to lower the chances of FDG the cancer returning. fluorodeoxyglucose AJCC fine-needle aspiration (FNA) American Joint Committee on Cancer A procedure that removes tissue samples with a very thin needle. biopsy A procedure that removes fluid or tissue genetic information samples to be tested for a disease. A chain of chemicals in cells that tells cells what to do. cancer stage A rating of the outlook of a cancer based on its hypopharynx growth and spread. The lowest part of the throat. carcinoma imaging A cancer of cells that line the inner or outer A test that makes pictures (images) of the surfaces of the body. insides of the body. chemotherapy IMRT Cancer drugs that stop the cell life cycle so intensity-modulated radiation therapy cells don’t increase in number. immunotherapy clinical stage A treatment with drugs that help the body find The rating of the extent of cancer before and destroy cancer cells. treatment is started. induction chemotherapy clinical trial The first treatment, consisting of cell-killing A type of research that assesses health tests or drugs, that is given to greatly reduce the extent treatments. of cancer. computed tomography (CT) lymph A test that uses x-rays from many angles to A clear fluid containing white blood cells. make a picture of the insides of the body. lymph node chemoradiation A small, bean-shaped disease-fighting A treatment of cell-killing drugs and high-energy structure. rays that are given during the same time period.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 42 Words to know magnetic resonance imaging (MRI) radiation therapy A test that uses radio waves and powerful A treatment that uses high-energy rays. magnets to make pictures of the insides of the radiologist body. A doctor who is an expert in reading imaging medical history tests. A report of all your health events and recurrence medications. The return of cancer after a cancer-free period. medical oncologist registered dietician A doctor who’s an expert in cancer drugs. A nationally-credentialed expert in food and metastasis diet. The spread of cancer from the first tumor to a side effect new site. An unhealthy or unpleasant physical or nasopharyngoscopy emotional response to treatment. A procedure to see the upper throat with a skull base device that is guided through the nose. A group of bones and tissues between the nasopharynx bottom of the brain and the structures of the The part of the throat that is behind the nose. face. neck dissection supportive care An operation that removes lymph nodes and Health care that includes symptom relief but not other tissue in the neck area. cancer treatment. Also called palliative care. oropharynx surgery The middle part of the throat. An operation to remove or repair a part of the body. pathologist A doctor who’s an expert in testing cells and surgical margin tissue to find disease. The normal-looking tissue around a tumor that was removed during an operation. PEG percutaneous endoscopic gastrostomy systemic therapy A type of treatment that works throughout the physical exam body. A study of the body by a health expert for signs of disease. targeted therapy A drug treatment that impedes the growth positron emission tomography (PET) process specific to cancer cells. A test that uses radioactive material to see the shape and function of body parts. prognosis The likely course and outcome of a disease based on tests.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 43 NCCN Contributors

NCCN Contributors

This patient guide is based on the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Head and Neck Cancers. It was adapted, reviewed, and published with help from the following people:

Dorothy A. Shead, MS Erin Vidic, MA Tanya Fischer, MEd, MSLIS Susan Kidney Director, Patient Information Medical Writer Medical Writer Design Specialist Operations Rachael Clarke Kim Williams Laura J. Hanisch, PsyD Senior Medical Copyeditor Creative Services Manager Medical Writer/Patient Information Specialist

The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Head and Neck Cancers, Version 3.2019 were developed by the following NCCN Panel Members:

David G. Pfister, MD/Chair Robert L. Foote, MD James W. Rocco, MD, PhD Memorial Sloan Kettering Cancer Center Mayo Clinic Cancer Center The Ohio State University Comprehensive Cancer Center - James Cancer Hospital Sharon Spencer, MD/Vice-Chair Maura L. Gillison, MD, PhD and Solove Research Institute O’Neal Comprehensive The University of Texas Cancer Center at UAB MD Anderson Cancer Center Cristina P. Rodriguez, MD Fred Hutchinson Center/ David Adelstein, MD Robert I. Haddad, MD Seattle Cancer Care Alliance Case Comprehensive Cancer Center/ Dana-Farber/Brigham and Women’s University Hospitals Seidman Cancer Cancer Center Jatin P. Shah, MD, PhD Center and Cleveland Clinic Taussig Memorial Sloan Kettering Cancer Center Cancer Institute Wesley L. Hicks, Jr., MD Roswell Park Cancer Institute * Randal S. Weber, MD Douglas Adkins, MD The University of Texas Siteman Cancer Center at Barnes- Ying J. Hitchcock, MD MD Anderson Cancer Center Jewish Hospital and Washington Huntsman Cancer Institute University School of Medicine at the University of Utah Gregory Weinstein, MD Abramson Cancer Center David M. Brizel, MD Antonio Jimeno, MD, PhD at the University of Pennsylvania Duke Cancer Institute University of Colorado Cancer Center Matthew Witek, MD Barbara Burtness, MD Debra Leizman, MD University of Wisconsin Yale Cancer Center/Smilow Cancer Hospital Case Comprehensive Cancer Center/ Carbone Cancer Center University Hospitals Seidman Cancer Paul M. Busse, MD, PhD Center and Cleveland Clinic Taussig Frank Worden, MD Massachusetts General Hospital Cancer Institute University of Michigan Rogel Cancer Center Cancer Center Ellie Maghami, MD * Sue S. Yom, MD, PhD Jimmy J. Caudell, MD, PhD City of Hope National Medical Center UCSF Helen Diller Family Moffitt Cancer Center Comprehensive Cancer Center Loren K. Mell, MD Anthony J. Cmelak, MD UC San Diego Moores Cancer Center Weining Zhen, MD Vanderbilt-Ingram Cancer Center Fred & Pamela Buffett Cancer Center Bharat B. Mittal, MD * A. Dimitrios Colevas, MD Robert H. Lurie Comprehensive Cancer Stanford Cancer Institute Center of Northwestern University NCCN Staff David W. Eisele, MD Harlan A. Pinto, MD Jennifer Burns The Sidney Kimmel Comprehensive Stanford Cancer Institute Manager, Guidelines Support Cancer Center at Johns Hopkins John A. Ridge, MD, PhD Susan Darlow, PhD Moon Fenton, MD, PhD Fox Chase Cancer Center Oncology Scientist/Medical Writer The University of Tennessee Health Science Center * Reviewed this patient guide. For disclosures, visit NCCN.org/about/disclosure.aspx.

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 44 NCCN Cancer Centers

NCCN Cancer Centers

Abramson Cancer Center The Sidney Kimmel Comprehensive St. Jude Children’s Research Hospital at the University of Pennsylvania Cancer Center at Johns Hopkins The University of Tennessee Philadelphia, Pennsylvania Baltimore, Maryland Health Science Center 800.789.7366 410.955.8964 Memphis, Tennessee pennmedicine.org/cancer hopkinskimmelcancercenter.org 888.226.4343 • stjude.org 901.683.0055 • westclinic.com Fred & Pamela Buffett Cancer Center Robert H. Lurie Comprehensive Omaha, Nebraska Cancer Center of Northwestern Stanford Cancer Institute 800.999.5465 University Stanford, California nebraskamed.com/cancer Chicago, Illinois 877.668.7535 866.587.4322 cancer.stanford.edu Case Comprehensive Cancer Center/ cancer.northwestern.edu University Hospitals Seidman Cancer UC San Diego Moores Cancer Center Center and Cleveland Clinic Taussig Mayo Clinic Cancer Center La Jolla, California Cancer Institute Phoenix/Scottsdale, Arizona 858.657.7000 Cleveland, Ohio Jacksonville, Florida cancer.ucsd.edu 800.641.2422 • UH Seidman Cancer Center Rochester, Minnesota uhhospitals.org/services/cancer-services 800.446.2279 • Arizona UCSF Helen Diller Family 866.223.8100 • CC Taussig Cancer Institute 904.953.0853 • Florida Comprehensive Cancer Center my.clevelandclinic.org/departments/cancer 507.538.3270 • Minnesota San Francisco, California 216.844.8797 • Case CCC mayoclinic.org/departments-centers/mayo- 800.689.8273 case.edu/cancer clinic-cancer-center cancer.ucsf.edu

City of Hope National Medical Center Memorial Sloan Kettering University of Colorado Cancer Center Los Angeles, California Cancer Center Aurora, Colorado 800.826.4673 New York, New York 720.848.0300 cityofhope.org 800.525.2225 coloradocancercenter.org mskcc.org Dana-Farber/Brigham and University of Michigan Women’s Cancer Center Moffitt Cancer Center Rogel Cancer Center Massachusetts General Hospital Tampa, Florida Ann Arbor, Michigan Cancer Center 800.456.3434 800.865.1125 Boston, Massachusetts moffitt.org rogelcancercenter.org 877.332.4294 dfbwcc.org The Ohio State University The University of Texas massgeneral.org/cancer Comprehensive Cancer Center - MD Anderson Cancer Center James Cancer Hospital and Houston, Texas Duke Cancer Institute Solove Research Institute 800.392.1611 Durham, North Carolina Columbus, Ohio mdanderson.org 888.275.3853 800.293.5066 dukecancerinstitute.org cancer.osu.edu University of Wisconsin Carbone Cancer Center Fox Chase Cancer Center O’Neal Comprehensive Madison, Wisconsin Philadelphia, Pennsylvania Cancer Center at UAB 608.265.1700 888.369.2427 Birmingham, Alabama uwhealth.org/cancer foxchase.org 800.822.0933 uab.edu/onealcancercenter Vanderbilt-Ingram Cancer Center Huntsman Cancer Institute Nashville, Tennessee at the University of Utah Roswell Park Comprehensive 800.811.8480 Salt Lake City, Utah Cancer Center vicc.org 877.585.0303 Buffalo, New York huntsmancancer.org 877.275.7724 Yale Cancer Center/ roswellpark.org Smilow Cancer Hospital Fred Hutchinson Cancer New Haven, Connecticut Research Center/Seattle Siteman Cancer Center at Barnes- 855.4.SMILOW Cancer Care Alliance Jewish Hospital and Washington yalecancercenter.org Seattle, Washington University School of Medicine 206.288.7222 • seattlecca.org St. Louis, Missouri 206.667.5000 • fredhutch.org 800.600.3606 siteman.wustl.edu

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 45 Index

Index

biopsy 17 cancer stage 21–22 chemoradiation 10, 22–25, 30–31 chemotherapy 10, 22–24, 30–31 clinical trial 9, 23–24, 30–31 computed tomography (CT) 16–17 distress screening 14–15 head and neck exam 14 imaging 16–17, 28 immunotherapy 10, 22, 25, 30 magnetic resonance imaging (MRI) 16 medical history 13, 28 nasopharyngoscopy 15 NCCN Cancer Centers 45 NCCN Contributors 44 neck dissection 10, 26–27 positron emission tomography (PET) 17 physical exam 14, 26, 28 radiation therapy 9–10, 22–25, 30–31 second opinion 39 shared decision-making 34 side effect 26, 29 supportive care 26 targeted therapy 10, 22, 25, 30

NCCN Guidelines for Patients®: Nasopharyngeal Cancer, 2019 46 Ü NCCN GUIDELINES FOR PATIENTS®

Nasopharyngeal Cancer

2019

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